ISSN 1006-298X      CN 32-1425/R

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肾脏病与透析肾移植杂志 ›› 2014, Vol. 23 ›› Issue (5): 496-500.

• 论文 • 上一篇    

反复黄疸、上腹痛伴肾病综合征

  

  • 出版日期:2014-10-28 发布日期:2014-11-03

Refractory jaundice、upper abdominal pain and nephrotic syndrome

2   

  • Online:2014-10-28 Published:2014-11-03

摘要:

摘要 33岁男性,胰、胆管、胆囊、肺和肾脏等多系统受累,哮喘病史多年。消化系统有梗阻性黄疸,伴腹部疼痛,CT示胰腺炎,胰腺弥漫性肿大,胰腺病理示浸润细胞少,但可见纤维化;胆囊活检病理示IgG4阳性浆细胞大于50个/HP。肾脏病表现为肾病综合征,肾功能正常,肾活检示肾小球膜性病变。血清IgG4水平明显升高,最终诊断为IgG4相关性疾病。

关键词: IgG4相关疾病, 肾小球膜性病变, 肾活检

Abstract:

ABSTRACT A 33-year-old man with multiple organ systems involvement, including the pancreas、cholecyst、cholangitis、lung and kidney , were admitted to our hospital. The disorders developed in kidney were nephrotic syndrome with normal glomerular filtration, the renal biopsy showed membranous nephropathy. The disorders developed in digestive system were obstructive jaundice an refractory upper abdominal pain. Cross-sectional imaging of abdomen showed diffuse pancreatic enlargement, pathological manifestations were few infiltrated lymphocytes with obvious fibrosis. The cholecyst biopsy showed that IgG4- positive plasma cells were over 50 every high power field. Bronchial asthma was last for many years. Evidently elevated serum IgG4 concentrations were detected. Hence, combining the clinical manifestation、elevated serum IgG4 concentrations and the histopathologic features, a diagnosis of IgG4-related disease was reached.

Key words: IgG4-related disease, membranous nephropathy, renal biospy